What is the clinical evidence from this study?

Study design: Cohort. Population: Adrenocortical Carcinoma (ACC) (n=124). Intervention: Referral to ENDOCAN-COMETE network at diagnosis (R-ACC) vs. Not referred or referred late (nR-ACC). Primary outcome: Overall survival in localized stage I-II ACC (5-year rate) (HR 3.9, 95% CI 1.2-13.0, p=0.026).

What question did this study set out to answer?

Determine the overall survival impact of early referral to the ENDOCAN-COMETE network for adrenocortical carcinoma patients.

May 17, 2026Open Access

Impact of the French ENDOCAN–COMETE Network on Overall Survival in Rare Adrenocortical Carcinomas

Key Result

Early referral to the specialized ENDOCAN-COMETE network significantly improved overall survival in patients with localized stage I-II adrenocortical carcinoma compared to non-referral.

Key Points

Determine the overall survival impact of early referral to the ENDOCAN-COMETE network for adrenocortical carcinoma patients.
Identified patients diagnosed with ACC from 2010 to 2017 using the French Network of Cancer Registries.
Categorized patients into referred at diagnosis (R-ACC) and not referred or referred late (nR-ACC) groups.
Compared median overall survival and survival rates at 1, 5, and 10 years between groups, adjusting for prognostic parameters.
Among localized stage I-II ACC patients, 1-year OS was 92% for R-ACC vs. 85% for nR-ACC; 5-year OS was 81% vs. 55%; 10-year OS was 75% vs. 35%.
The survival advantage for R-ACC group was significant (hazard ratio 3.9, P=0.026) for localized stages.
No significant difference in overall survival rates was observed for advanced ACC patients between the two groups.

Study Design

Type

Cohort (n=124)

Multicenter

Yes

Structured PICO

Does early referral to the ENDOCAN-COMETE network improve overall survival in patients with adrenocortical carcinoma?

Population

124 patients diagnosed with adrenocortical carcinoma (ACC) between 2010 and 2017 from the French Network of Cancer Registries (FRANCIM), mean age 53.6 years, female-to-male ratio 2:1.

Intervention

Referral to the ENDOCAN-COMETE network at diagnosis (R-ACC)

Comparator

Not referred, or referred late to the ENDOCAN-COMETE network (nR-ACC)

Outcome

Median overall survival (OS) and OS rates at 1, 5, and 10 yearshard clinical

Early referral to a specialized national network significantly improves overall survival in patients with localized stage I-II adrenocortical carcinoma.

Main Result

Effect estimate: HR 3.9 (95% CI 1.2-13.0)

Absolute Event Rate: 81% vs 55%

p-value: p=0.026

Limitations

7% of patients were excluded due to lack of precise diagnosis or missing information on their health care pathway
Registries covered only 18% of the French territory, which may not fully reflect other regions
FRANCIM registries do not reliably capture information on recurrence
Absence of referral to expert centers does not necessarily imply that patients did not receive a second opinion (e.g., through a national web-based meeting)

Abstract

BACKGROUND: Adrenocortical carcinoma (ACC) is a rare cancer. The French ENDOCAN-COMETE network was established in 2009 to coordinate care locally and nationally, and to promote research and education on malignant adrenal tumors. Evidence demonstrating the benefits of this organization is currently lacking. PATIENTS AND METHODS: Patients diagnosed with ACC between 2010 and 2017 were identified from the French Network of Cancer Registries (FRANCIM). Patients were categorized based on referral to the ENDOCAN-COMETE network as either referred at diagnosis (R-ACC) or not referred, or referred late (nR-ACC). Median overall survival (OS) and OS rates at 1, 5, and 10 years were compared between the R-ACC and nR-ACC groups after adjustment for prognostic parameters. RESULTS: A total of 134 patients with ACC were identified from the FRANCIM registries, corresponding to an incidence of 1.4 cases per million person-years. Ten patients were excluded because of insufficient information regarding their health care pathway. The final analysis included 124 patients (mean age, 53.6 years; female-to-male ratio, 2:1). At diagnosis, 45.2% had European Network for the Study of Adrenal Tumours (ENSAT) stage I-II disease, 48.4% had stage III-IV disease, and 6.4% had an unknown stage; endocrine and/or tumour-related symptoms were present in 62.9% of patients. Among the analyzed patients, 87 (70%) were R-ACC, whereas 37 (30%) were nR-ACC. In patients with localized stage I-II ACC, OS rates were significantly higher in the R-ACC group than in the nR-ACC group: 1-year OS was 92% versus 85%, 5-year OS was 81% versus 55%, and 10-year OS was 75% versus 35%, respectively. This survival advantage remained significant after adjustment for prognostic factors (hazard ratio, 3.9; P=.026). In contrast, among patients with advanced ACC, OS rates were similar between the 2 groups. CONCLUSIONS: Our study demonstrates an OS benefit for patients with stage I-II ACC who were referred early to specialized centers within the ENDOCAN-COMETE network. The combined use of the French cancer registries and dedicated national networks provides the strongest evidence to date demonstrating the impact of such a network.

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